The laminopathy Hutchinson–Gilford progeria syndrome (HGPS) is caused by the mutant lamin A protein progerin and leads to premature aging of affected children. Despite numerous cell biological and biochemical insights into the basis for the cellular abnormalities seen in HGPS, the mechanism linking progerin to the organismal phenotype is not fully understood. To begin to address the mechanism behind HGPS using Drosophila melanogaster, we have ectopically expressed progerin and lamin A. We found that ectopic progerin and lamin A phenocopy several effects of laminopathies in developing and adult Drosophila, but that progerin causes a stronger phenotype than wild-type lamin A.
Conference Article| November 19 2008
Towards a Drosophila model of Hutchinson–Gilford progeria syndrome
Gemma S. Beard;
Joanna M. Bridger;
Ian R. Kill;
David R.P. Tree
David R.P. Tree 1
1Centre for Cell and Chromosome Biology, Division of Biosciences, School of Health Sciences and Social Care, Brunel University, Uxbridge, Middlesex UB8 3PH, U.K.
1To whom correspondence should be addressed (email firstname.lastname@example.org).
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Gemma S. Beard, Joanna M. Bridger, Ian R. Kill, David R.P. Tree; Towards a Drosophila model of Hutchinson–Gilford progeria syndrome. Biochem Soc Trans 1 December 2008; 36 (6): 1389–1392. doi: https://doi.org/10.1042/BST0361389
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